Clinical and laboratory manifestations of Kikuchi's disease in children and differences between patients with and without prolonged fever

Background: Kikuchi's disease (KD) is characterized by cervical lymphadenopathy with or without fever. It has been recognized worldwide but seldom reported in pediatric patients. Methods: From January 1985 through December 2001, 64 patients younger than 18 years of age with pathologic proof of KD were enrolled in this study. The clinical manifestations, laboratory data and outcomes were reviewed. Results: There were 35 male patients and 29 female patients with age ranging from 2 to 18 years and a median age of 16. All patients had cervical lymphadenopathy except 1 who had generalized lymph-adenopathy. Lymph nodes of 32 patients (50%) were painful or tender or both. Lymphadenopathy was unilateral in 52 patients (82.5%). Lymphadenopathy associated with fever was observed in 21 patients (32.8%). Other signs such as skin rash, hepatomegaly or body weight loss were less common. Twenty-six patients (40.6%) had leukopenia and 2 patients had leukocytosis. Nearly one-fourth of the patients had mild liver dysfunction. Virologic or immunologic studies were normal in most patients. Patients with prolonged fever were more likely to have leukopenia (P < 0.05). All patients recovered, but 1 developed systemic lupus erythematosus 5 years later, and the other had vasculitis syndrome 2 years later. Conclusions: The clinical presentation of KD in pediatric patients is similar to that of adults. KD is a benign, self-limiting disease; prolonged fever occurred only in 32.8% of pediatric patients in our cohort. Leukopenia was the only feature significantly associated with prolonged fever.